Last week was a very busy week in the EB community. Many of the final springtime fundraisers took place including Jogging for Jonah, Butterfly Wishes for Ellie, Love for Lucas and the sixth annual Walk for EB in Cincinnati. As summer approaches, we thought it would be a good time to highlight those events that we have learned about taking place later this year, so far, and hopefully inspire others to coordinate something in their own communities.

If you hear about any other events or are planning one yourself, please send us all of the details. You can email me at leslie.rader@hollisterwoundcare.com. We’ll do our part to help spread the word here at the blog and via Twitter and Facebook.

• Ayden Lee’s 3rd Birthday Party (Niles, OH)
  This special Elmo-themed birthday celebration is being held on Saturday, June 15. The event will take place at Waddell Park, 1000 W. Park Av. Guests are asked to bring a covered dish and drinks, as well as wear the color red in honor of Ayden’s best friend Jaxon who has EB. In lieu of birthday gifts, donations in Jaxon’s name are requested at www.debra.org/donate to benefit families supported through DebRA of America. For more information, click on this event link.
   
• MW Foundation and DebRA Tennis Pro-Am (Queens, NY)
  Details for this sporting event are still being worked out but the scheduled date is Wednesday, August 28 and plans are for West Side Tennis Club, located at 1 Tennis Place, to host the event. Proceeds from this inaugural fundraiser will benefit DebRA of America. For more information, click on this event link and scroll halfway down the page.
   
• 2013 Medtronic Twin Cities Marathon Weekend (Minneapolis, MN)
  The Jackson Gabriel Silver Foundation is currently recruiting for its team as part of a weekend-long array of events Friday through Sunday, October 4-6 including a marathon, 10-mile, 10K and 5K. Team JGSF will be running to support the work of University of Minnesota EB researcher Dr. Jakub Tolar. If you would like take part as a member of the team or get involved in some way, email alevine@jgsf.org. Proceeds will benefit DebRA. For more information about this and other events for the organization, click on this event link.
   
• Frontiers Day Fundraiser (Paris, AR)
  This event coincides with the 33rd Annual Mt. Magazine Frontier Days Festival held in October, featuring its first ever Civil War re-enactment. The EB event will be held Saturday, October 5 at the Logan County Fairgrounds, 301 W. Walnut. There will be a DebRA booth with information about EB and raffles, giveaways and a special silent auction of handcrafted items. You can get additional information about this year’s event or how to donate an item by emailing Cindi Hunter at meadowfairies@gmail.com. Proceeds will benefit DebRA. For more information, click on this event link.

Mother’s Day is just around the corner. This is often a time when children and spouses pull out all the stops to show they care. For moms of children with EB, being a mom carries a whole different set of challenges and stresses.

We thought this would be a great opportunity to offer some supportive suggestions to those who may know an EB mom personally and want to remember them at the holiday. Remembering her with a present is nice but helping her find a way to use the gift and enjoy it may take some extra effort on your part. Remove any guilt she may feel for allocating time to herself by making your present stress-free. Here are some gift ideas that may offer a small lift of spirits and demonstrate you care.

• Offer A Fresh Idea. Consider fresh-baked goods, a fresh fruit arrangement or fresh flowers. All moms (and some dads, too) can find themselves running around as they manage multiple lives’ daily routines, but it can be a wonderful pick-me-up to receive a reminder from a good friend or co-worker to stop for a moment and savor something new, fresh and handpicked.
   
• Encourage Downtime. Everyone knows that devoted moms tend to forget to take a little time for themselves. It’s not always easy to carve out personal time, but perhaps a reminder like a gift card for downloadable music for her mp3 player or downloadable books for an e-reader is a thoughtful way to say ‘it’s okay for you to steal away for a few moments, too.'
   
• Lend a Hand. One thing that can be difficult for EB moms is finding a window of opportunity to step away from the house and run errands. Offer to fill in at a set time on the same day weekly for a month (or more) as a gift that truly keeps on giving.

• Rejuvenate. The idea of a day of pampering whether it’s a much needed replenishing of one’s mind and body at a spa or a simple afternoon getting a manicure may sound attractive to a hard-working mother. To an EB mom, it may be just what she needs in that stressful moment to catch herself from overworking herself so hard she jeopardizes her own health, restfulness and clarity.

• Whisk Away. What better way to step away for a few moments than a night out for dinner and a movie with a good friend or a special afternoon shopping, taking in a local attraction or simply lounging by the community clubhouse pool with a friend and a good book. Chat with a spouse or family member about relieving mom of her responsibilities for a short period of time and snag her for a little fun time away from home to catch up and unwind.

One study is not yet recruiting and the status of two studies is currently labeled unknown. Of those open, recruiting EB research studies, eight studies are taking place in the U.S. and four are being led outside of the U.S.

The following briefs highlight general aspects of the U.S. studies currently recruiting. For the latest detailed information about all EB studies open and recruiting here in the U.S. and abroad, please visit the NIH’s Clinical Trials page at the following link: http://clinicaltrials.gov/ct2/results?term=epidermolysis+bullosa&recr=Open.

The following is not an endorsement but merely a brief summary of the EB clinical trials available at this time and is for informational purposes only. You should visit the relevant link below for more information. You should also consult your health care professional and thoroughly understand the potential risks involved with a clinical trial before you participate in any clinical trial. We take no responsibility for any results or outcomes associated with the following clinical trials.

• Gene Transfer for Recessive Dystrophic Epidermolysis Bullosa. Dr. Alfred T. Lane of Stanford University’s School of Medicine leads this study first launched in December 2010. The trial is being conducted in collaboration with the National Institute of Arthritis and Musculoskeletal and Skin Diseases. This study involves researchers creating a graft of the participant’s skin that has been genetically engineered in a culture, in an attempt to correct its cellular protein deficiency causing RDEB. Investigators then transplant the “corrected” cells back onto the patient’s skin. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Stem Cell Transplant for Epidermolysis Bullosa. This study by the Masonic Cancer Center at the University of Minnesota, is attempting to test its primary hypothesis that the infusion of bone marrow or umbilical cord blood from a healthy donor will correct certain deficiencies and reduce the skin fragility characteristic of several severe forms of EB. EB patients are being treated with a combination of a chemotherapy regimen, a bone marrow or umbilical cord blood stem cell transplant and a mesenchymal stem cell transplant. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Characteristics of Patients with Dystrophic Epidermolysis Bullosa. In addition to its gene transfer study for RDEB patients, Stanford University is conducting a screening of subjects with DEB to evaluate the characteristics of these patients, who often develop severely painful blistering and open wounds, and examine their cells as a means to support the development of future therapies. For more information about this study, click here to link to ClinicalTrials.gov.
   
• A Study of the Efficacy and Safety of ABH001 in the Treatment of Patients with Epidermolysis Bullosa Who Have Wounds That Are Not Healing. Launched in December 2012, this trial by Shire Regenerative Medicine will attempt to evaluate the efficacy and safety of ABH001 for treating EB patients with wounds that are not healing. The hypothesis being tested is that ABH001 may initiate and continue the wound healing process for these particular EB patients. Currently, a handful of sites in the U.S. are recruiting for this study including UCSD Children’s Hospital in San Diego, Northwestern University in Chicago and Virginia Clinical Research in Norfolk, Va. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Treatment of Chronic and Non-Chronic Wounds in Patients with Recessive Dystrophic Epidermolysis Bullosa Using Helicoll Collagen Dressings Versus Standard Care. This trial is being led by Dr. Lane at Stanford University and launched last October. Its purpose is to test the effectiveness of a specific collagen wound dressing (Helicoll) against traditional wound dressing for treating chronic and no-chronic wounds of RDEB patients. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Characteristics of Adult Patients with Recessive Dystrophic Epidermolysis Bullosa. Stanford University is also conducting a screening of RDEB patients similar to its Dystrophic EB trial, in particular those adults who survive with the diagnosis into adulthood, to evaluate their characteristics. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Efficacy of Granulocyte Colony Stimulating Factor in Patients with Dystrophic Epidermolysis Bullosa. This feasibility study from Vanderbilt University in Nashville, TN launched in February 2012 and attempts to measure the effectiveness of Granulocyte Colony Stimulating Factor treatment for DEB patients in a seven day course of treatment. Follow-up with the patients will take place after seven days and 30 days following discontinuation of the drug. For more information about this study, click here to link to ClinicalTrials.gov.
   
• Grafting of Epidermolysis Bullosa Wounds Using Cultured Revertant Autologous Keratinocytes. This is another study being led by Dr. Lane at Stanford that launched in fall 2011. Revertant Mosaicism means that a patient has two different genetically different cell populations due to spontaneous mutations, and for this study, the research team is attempting to use such circumstances to treat a patient with his or her own normal, non-fragile skin patches. That is, the team will try to culture cells from these areas the EB patients’ skin to create grafts for the wounded areas of the same patient. For more information about this study, click here to link to ClinicalTrials.gov.

Over the years, I have raised in excess of $100,000 to create and support this important program for all the families that have recently received news of an EB diagnosis. Starting and running this program has allowed me to meet with new families in our community and offer them support in the way of supplies, information and access to other resources for which they might have an immediate need. I am proud of this program and the way it has developed over time.

In January of this year, my role with DebRA expanded. I transitioned from serving as a member of DebRA’s Board of Trustees to being elected to the role of President by my peers. My term is for two years, and if you know me well, then you know that I’m excited to play a bigger role.

Recently, I determined that I was not going to be able to coordinate my annual Butterfly Fashion Show and Luncheon fundraiser. To be honest, I have been feeling down about that because it felt like I was failing as an EB parent, which I will always consider myself. When the call to play a larger, more influential role presented itself, I couldn’t have been happier. Becoming President of the Board of DebRA has given me a renewed sense of purpose.

This is a great opportunity to do more for an organization that I truly believe in. My whole mission in life is to help this organization, because it was the first to reach out to me when my own daughter was born. I want to do what I can to help make this organization even stronger. DebRA provides many services to support EB families, but it’s my job as well as that of the Board of Trustees and the organization’s executive director to find additional ways that they can provide guidance and resources where needed as well as encourage researchers to continue their quest for a treatment and a cure for all forms of EB.

So what does this really mean as far as my focus and responsibilities? I will be taking part in monthly executive committee meetings and communicating regularly with DebRA’s executive director Brett Kopelan to stay current on the day-to-day operations of the organization. I also look forward to continuing to create and grow working committees that can define goals and objectives to contribute to the betterment of the entire organization. As President of the Board, I feel my main job is to manage the Board of Trustees and to act as the lead in making sure that DebrRA operates in the best interest of the EB community. I have faith in our executive director, and the entire Board of Trustees is committed to support him and the organization as a whole. Perhaps not so coincidentally, Brett Kopelan is the Father of an RDEB little girl and served on the Board of Trustees with me before he assumed the role of Executive Director.

Several working committees are already hard at work, like the Family Crisis Fund committee, but there is other work to be done, more committees to form and I’m eager to play a part in strategizing and implementing these initiatives.

I will continue to use the EB Resource platform as a means to keep families in the loop on not only what DebRA is doing or has in the works, but also on what others in the community are working on, dealing with or tackling, as this information becomes available to share. If you have any questions about my new role or your own thoughts on ways the organization can provide additional support, please feel free to email me at leslie.rader@hollisterwoundcare.com. I enjoy hearing from you.

For families living with epidermolysis bullosa, the pains that accompany it far surpass the physical and emotional impact. EB takes a tremendous toll financially on the family, too. This may not be something that others just learning about the disease may realize, so it’s our responsibility as EB advocates out in the community to share the full story.

Economically, EB hits us as families from every possible direction – medical bills, additional caregiver support, daily wound care supplies, the need to purchase special clothing or EB-friendly accessories or toiletries as opposed to what’s sitting on the clearance table at the local department store.

There are many things an EB family cannot do automatically or without careful forethought to ensure the safety and comfort of the person with EB. That inability to simply act on the cheapest alternative can be costly, whether you’re seeking out hard-to-find garments without buttons, elastic and scratchy zippers or trying to find special footwear.

What would you estimate your family pays in wound care materials on a monthly basis? For many of the families I talk to in the community, this figure can range anywhere from $5,000 to $10,000, or greater. Medical bills can drain families of thousands of dollars over the course of several months or a year. The impact that figures like these can take on a family’s budget is something that even those who may not fully comprehend EB could identify with.

Those of us directly faced with EB or even those who know of a neighboring family affected have an opportunity to educate others not only on the physical and emotional scars EB leaves, but the economic devastation the skin disorder inflicts. As you coordinate EB awareness events and fundraisers, find ways that you can convey this message, as well, to those who are first learning about EB.

Consider sharing this kind of information with other families during your educational process. The supporters you are seeking will already understand the costs associated with raising a family, but are more likely to empathize when they see the full impact EB takes on a family’s entire well-being.

*Jessica Kenley is a mom and author of KIDOWED, a book in which she shares her own personal account of her family’s experience with EB and the impact of losing two of her children to the skin disorder. She was recently featured in a two-part Q&A series. Click here to read part I and part II of that interview.

This entry is about my experience in publishing a book about my life in a two-year snapshot for the world to see—an e-book—but still a book.

I started writing a diary every day on the sage advice of an amateur grief counselor. I should clarify—she was not an amateur at grief, having lost a husband who she had had more years on earth with than not, but possibly an amateur at counseling. Of the variety who thinks the glass is always half full if you look at it in the right light and that we of the grieving community should discard our negative thoughts and only voice and allow ourselves to think the positive ones. She didn’t quite know what to do with me.

Most of the people in our group were older women who had lost husbands of your everyday garden variety diseases of old age —cancer being the most common. I should point out that I am not discounting or minimizing their grief at all, but I did find it difficult to relate to them. I was the youngest in the group by at least 25 years and the only one who was there as the result of losing a child—well on my way to two, for that matter. I didn’t have any positive thoughts. I was furious. We eventually started having “individual sessions” and she told me one day after listening to me rant over iced teas for a while that I was going to “burn down the world” someday. I still don’t know what that means, but I think she meant it in a good way, as if I had the potential to change the things about the world that I didn’t like. In any case, that’s how the book began. Eventually it turned into something that I was writing as if I was talking to someone else, if only because I didn’t have anyone to talk to in such a frank and honest manner about what was running through my head on a constant basis, especially while I was watching my daughter die in front of me every second of every day while simultaneously knowing what was going to happen to her and being absolutely and completely helpless to stop it.

The bottom line is that the book is brutal to read. It asks people of all walks of life to stand beside me through my hellish experience and endure the aftermath, too. It is not an easy read. It wouldn’t even be an easy read if it were fiction. I wasn’t thrilled with the idea of people I didn’t know reading extremely personal and potentially humiliating things about me (such as my dating and drinking habits) while I was trying to cope with the deaths of my children, but I did feel like the importance of EB awareness trumped any qualms that I might have about judgment from strangers, which is why I published it, and I would encourage others to do the same.

The response I have gotten from people who have read the book is overwhelmingly positive, with some negativity and judgment that I believe comes mostly from misunderstanding sprinkled in here and there, which is not so bad, all things considered. You can’t win ‘em all, as they say.

Here are the things I wish I would have known before I published the book or that I wish a friend might have told me, even if it was hard to hear:

1. You’re not likely to get rich or famous. Not even close, especially with an e-book. Authors who make a living from writing do it for a living, all the time, every day, and fail continuously before success, even if they have something that is extremely important to say, and these are the ones with advertising and agents.
2. People will judge you relentlessly and harshly, if only because they cannot identify with your situation. Do NOT, under any circumstances, reply or comment on negative reviews of your book. It makes the author appear petty and unprofessional. On the flip side, DO encourage people who liked the book to post positive reviews. The sad fact is that people who don’t like it are much more likely to take the time to review it than people who do, so when people contact you telling you how much they like it, tell them to post a review.

Here is what I can tell you…

• If you think your book is worth publishing, I can guarantee you that a lot of other people will, too. You cannot fake passion or truth. If you truly believe that the world should know about it, then say it. Really, what do you have to lose?
• You will most likely create a following of people who are passionate about what you publish, and even if it’s just a few people, who cares! Those are people that will spread the word where you couldn’t.

I published Kidowed because I thought that more people should know about EB. The e-book has been in existence only since July 2012, and about 10,000 people have read it. The vast majority got it for free, and I got some bad reviews, but I got many more good ones, and consider this—upwards of 8,500 of those people never knew EB existed before I published it, and now they do.

Because I published this book, I was afforded the opportunity to do a reading at a prestigious international conference that had doctors and researchers from 25 different countries who were all involved with EB in some way, which I count as the highlight of my career as an EB advocate. And my little e-book made it to #1 for free memoirs that weekend on Amazon.com, if only for a short time.

Also, I am currently a guest blogger on this fantastic website—would you have known that I existed if I hadn’t published a book? A difference is a difference, so don’t hesitate when given the opportunity to make one. You won’t regret it.

For parents of a child with EB, helping their child become acclimated to a new environment when he or she reaches school age can become their main mission.

It’s not uncommon for other parents, teachers or school administrators to initially raise questions about your child’s visible skin condition, particular when they most likely have never heard of EB nor understand it. There are some tools available and steps parents can take to help educate others and make the transition for their child as smooth as possible.

• If your child will be starting school next fall, you may want to inquire with DebRA about the organization’s classroom DVD “What Is Epidermolysis Bullosa (EB)?” The short film is also available online and clocks in just under six minutes. It uses an analogy of grass and its roots to explain in simple language how skin impacted by EB is unable to adhere to layers below it, unlike the way a lawn’s roots adhere to the earth below it. Your child’s classmates, teacher and their families also get to hear from students in one school talk about their interaction with a young classmate with EB and from students with EB themselves. They attempt to dispel myths associated with EB and share ways that they may need additional support from others around the classroom or at play. Click here to access the film online at the DebRA website.
• Some parents choose to schedule a meeting before the new school year with teachers and key administrators at the school. Such a conversation before the hectic pace of class assignments and activities can provide a great window of opportunity to educate them about the support a child with EB may need during the school day or extracurricular activities.
• Parents may also seek out meetings before the school year where parents are invited to visit the school for activity sign-up or other class-related discussions. It can be a helpful way to introduce the topic in a conversational, informal atmosphere before children begin interacting with one another.
• Some families have even developed a short half-sheet or one-sided page with information about EB that they then ask the teacher beforehand to distribute and send home with students on the first day, so that any potential questions or concerns can be addressed and also to encourage students in particular to support the teacher and classmate with EB wherever necessary.

If you are a parent who has already gone through the first day of school jitters with your child diagnosed with EB, what strategies and tips can you offer other parents to help their child’s transition to the classroom be a smooth, comfortable and safe one?

A lot of information gets communicated every day on Facebook and it’s very easy for helpful suggestions or supporting words to slip by without your knowledge. We want to encourage you to stop by our Facebook page on a regular basis and let your voice be heard within the community on those subjects, popping by with a question or commenting about whatever you feel most passionate.

** Please note that comments and recommendations are offered by individual members of the EB Resource Facebook community and are not necessarily recommended or endorsed by EB Resource. Individuals should always consult a physician before making any changes to their wound care program or other care regimens.

• We always like to pose questions to our community members to get their perspective on a variety of topics. In February, we asked EB Bloggers what personal benefits they’ve gained by being a regular blogger. Derra Nicole Sabo shared that for her, the benefits are two-fold: “Being able to not only help others, but to connect with other EBers.” If you’re an EB blogger, how would you answer that question? It’s not too late to share your thoughts here. We appreciate what EB bloggers do to encourage interaction among the EB community and spread the word outside of our immediate circle of those directly impacted. By being a loud, steady voice online that keeps conversation about EB flowing, you provide an invaluable service that can indirectly support us all.
   
• Earlier this year, we featured a two-part Q&A with Jennifer Gorzelany, a consultant to Shire (formerly Lotus Tissue Repair) who answered questions about the EBCare Registry. In late January, one member of the community, Sue Altinkeser, commented that there may be confusion among community members about who should actually take part in registering their family’s data: “People are thinking if they are not dystrophic then it’s nothing to do with them…it may be easier to re-word the registry so all are comfortable in adding their data.” In response to the concern she expressed, we reached out to Lotus and tried to clarify for members what the group’s expressed mission is. EB Resource commented: “The goal of the EBCare Patient Registry is to help all individuals with EB. It is important that the registry collects information from people with all forms of EB, so that as much data about EB is available to qualified researchers as possible. Lotus Tissue Repair is currently working on a treatment for DEB. In the future, however, Lotus Tissue Repair and other companies or researchers may use the information in the registry to study other forms of EB, hopefully leading to improvements in care and the development of new treatments. Every EB patient can help with this effort by joining!”
   
• Some members have found Facebook to be a helpful site to reach out for information or share news about an upcoming campaign or event. Rochi Orbegoso Pea introduced herself and daughter Raquel in November 2012 and their plans to launch an EB awareness campaign at her daughter’s school. Rochi was seeking a speaker at the time for an upcoming event, and we offered to send information on some prospective speakers in her area. Aaron Mavro let members know about a short film competition and his video entry focused on a talented young painter named Kate who has EB and serves as an ambassador for DebRA. Aaron shared the link so members could watch online and vote. Are there any upcoming events, campaigns or efforts you can share with the community and to which members can lend their support and encouragement in some way?
   
• Members of our community let their voices be heard in other ways on our Facebook page by letting us know what stories touch them most. We are always fascinated to see which stories receive the most “likes.” For example, some of you were pleased to learn about a southern Indiana high school who found a way to turn a much-anticipated conference game into an opportunity to educate audience members about EB. Others of you were inspired to “like” a story about 11-year-old Molly Gibbons whose fearlessness and boisterous personality are bigger than her petite frame. We wanted to make sure that we gave everyone advance notice of Rare Disease Day, held February 28, by sending out a reminder two weeks earlier in an update. Several of you indicated that you appreciated the early notice with a “like.” And when we featured part one of a special virtual conversation with EB advocate Jamie Silver of Jackson Gabriel Silver Foundation, you not only told us you liked it: you shared it with your other Facebook friends and we always love to see that!

We love hearing from you…however you choose to speak up in the EB Resource community. So join in the conversation and let your voice be heard, because we welcome it!

In this conclusion of the Q&A, Jessica talks about the outcomes of her experience and where she sees the greatest need within the EB community.

Q. EB Resource (EBR): What would you say is the overall message of your book, "KIDOWED?"
A. Jessica Kenley (Jessica): There are several messages that I think are extremely important that I tried very hard to convey with the book — the most critical one is that Epidermolysis Bullosa in all of its forms is a horrible disease that robs children and families of having a normal existence in multiple ways, and there is no good reason why more people shouldn’t know and care about it so that more money can be raised for better treatments and a cure. The "sub- messages" include ideas like how some people could be more sensitive to grieving parents, how the medical community could improve some of their practices (especially when it comes to honesty) with the parents of sick children and, with any luck, a message of hope to people who have lost someone very dear to them. It is possible to survive these things, even if one does get stuck in a stage of grief for a very long time.

Q. EBR: What has been the most unexpected outcome for you from writing the book?
A. Jessica: The most unexpected negative outcome of the book has been the judgment, rage and sometimes outright hatred that I got from some people, both people that know me and people that don’t, about my personal choices, my feelings and how I portrayed some people in the book, even though all the names were changed except for mine and my children’s. I never expected that the message that some people would get from the book would not be about spreading awareness about EB at all, but that I somehow was out to hurt people emotionally, even though I had made several disclaimers (both in the book, online and personally to people) about how the book is a journal and was not edited or censored in any way. I did not feel like the book should be censored, because it might take away some of the emphasis and meaning of my words. I did not want it to come across as "sugar-coated" in any way. I also was surprised that some people in my family stopped talking to me at all because they didn’t "want to be in my next book." Others asked me not to put pictures of them holding Ethan and Kaylee on the Kidowed Facebook page, because they wanted their privacy protected. That really surprised me.

I will always be extraordinarily proud that I was able to be Ethan and Kaylee’s mother, and that I got to be involved in their short lives to the extent that I did, and I guess I mistakenly assumed that other people in my family felt the same. Honestly, I have stalled on the second book because of these unexpected critical reactions. Obviously I knew that not everyone would like the book, but I did not predict some of the very hurtful reactions I got.

The most positive unexpected outcomes of the book have been the great many new friends that I have and old friends in the EB community that I have been given the opportunity to reconnect with. I was extremely honored to be invited personally by the President of DebRA Canada to speak at the DebRA International Congress in September of 2012, especially since the book had only been published in July and only in an online format. I also got a lot of help in spreading the word about the book from people in the EB community, many of whom I didn’t even know, which was a really wonderful surprise, since the book has never been formally advertised. I just cannot express how much it means to me when people take the time to message me or write lovely reviews out of the kindness of their hearts — or follow the Facebook page. It is really heartwarming and inspiring that the book has reached thousands of people. It makes me feel like it was well-worth it to publish it when someone who has never heard of EB reads it and then becomes involved in the cause. There’s just nothing like that kind of feeling. It makes me feel like I am doing my small part in spreading awareness.

Q. EBR: Since releasing your book, you’ve been very open in sharing opportunities for others to talk with you about your experience and have even made the book available on several occasions as a free e-book download. What were your reasons for offering free e-book downloads?
A. Jessica: I wanted to make sure that even people who maybe couldn’t afford the book could still have the opportunity to read it. I would never want it to be cost-prohibitive, and the point of the book was never to make money on it. It was and is to spread a very important message about a devastating and little-known disease.  This is why I have also put the Kindle Free Reader Apps link on the KIDOWED page several times. I want people who don’t have a Kindle to be able to read it too if they want to. The most successful free giveaway was the weekend of the DebRA International Congress, where in only two days, 5,360 people downloaded the book for free, and it actually made it to #1 on the Amazon site for memoirs, thanks to the advertising efforts of the wonderful people at DebRA Canada. It was simply amazing, and nothing could have made me happier. My dream is that one day soon EB will be a disease that the whole world knows about, so that we can finally have a cure. Too much time has gone by already without good treatments, especially for children whose families are underinsured or do not have health insurance. This has to change.

Q. EBR: Have you been involved with any specific EB-focused organizations or patient/family advocates to collaborate on educational campaigns or other efforts to raise awareness about EB? And if so, can you talk about those?
A. Jessica: I am on many EB-focused Facebook pages, and several personal pages or blogs of people who have EB or a child with it, and I try whenever I can to offer what I know about it when someone has a question about wound care or advocacy programs or really anything that I can help with. I have also recently gotten involved with Debrabox.com, which is out of Ireland, and is a new company that is just getting off the ground that will sell merchandise in order to raise money for EB charities. Unfortunately, I have only just recently become involved with this company, so I don’t have a whole lot of information about it, but I have high hopes for their efforts, especially since the man who founded it has a son with EB and is very motivated.

Q. EBR: From your own communication with EB-focused organizations and other families who have shared their stories, whether it’s through social media, blogging or talking with local media, where do you see the greatest needs of the EB community?
A. Jessica: The greatest need that I see is still money for struggling families, better insurance coverage and more patient advocates, especially in hospital settings. EB is an extremely expensive disease to treat, even with health insurance, which is not something that everyone realizes. When a child is diagnosed with EB, the entire family’s whole existence often becomes dealing with it, and things like traveling to doctor visits constantly can be taxing on even well-off families, especially if they need to go to an EB clinic that might be thousands of miles away and require a lengthy stay. This might also include hotel stays and large food bills, not to mention all of the specialized equipment that this might necessitate.

I hear heartbreaking stories often about families trying to raise money for a special seat, a wheelchair, a van or even actual wound care supplies that they just cannot afford. Other supplies that EB children and adults might need that are rarely covered by insurance include things like specialized feeders, special diapers and clothes, nutritional supplements. The list goes on and on. This should never happen. These families undergo so many hardships as it is that they just should never have to worry about being able to afford the best care possible for their children. My children both had two different insurance companies, and even eight years after Ethan was born, my finances have never recovered. It is also extremely difficult for single parents to afford caring for a child with EB, as working is often nearly impossible, and for people who have adopted or are in the process of adopting a child with EB, especially from a different country, because then they might not be eligible for health insurance for an extended period of time. And of course, God forbid the worst happens, funeral costs are very expensive too, as are headstones. The cost of this is also something a parent should never have to worry about, as they are already enduring the most painful thing any parent can.  I’ve even read one particularly heart-wrenching story about a child who died at a hospital that was very far away from the family’s home, and they had to have a fundraiser in order to transport the child’s body home and to pay for funeral costs. How awful.

The other great need, as I mentioned, is more patient advocates. The ones that I know of are fantastic people who seem to literally eat, sleep and breathe EB, and they do a WONDERFUL job, but there just aren’t enough of them. I realize that this must be a very taxing job to have emotionally, physically and financially, and my guess would be that there aren’t more very active EB advocates, because they probably suffer from "burnout" or "compassion fatigue," such as is common in health care and social work. My dream job would be one that entailed going around to hospitals that currently are treating an EB patient and stay at the hospital with them to support the families and the patients and educate the medical staff on how to best care for an EB patient and also to give the parents "breaks" to sleep or eat or get out for a while without having to worry about their child. I would have loved it if someone was there for me when my son was in the hospital who knew better how to handle the medical professionals and also the basic "ins and outs" of the health care system. Studies have shown over and over that any patient gets better care when they have a strong advocate and goodness knows that these families can never have too much help or support.

Q. EBR: If somebody out there reading this interview feels inspired by your story and wants to do something similar by writing their own book, what advice can you offer them?
A. Jessica: Even though it wasn’t my first choice, I actually would recommend putting a book online first to gauge the reaction and success before spending the money to advertise and having to go through the many inevitable and discouraging rejections from publishers. Part of the reason I hadn’t published my book sooner is that I didn’t have the resources, and had I known how fast and easy it is to publish online, I would have done it much sooner. I still do hope to publish "KIDOWED" and its sequel in print, but the online publishing option was really a blessing to me. My other bit of advice would be to "Just Do It!" I agonized and put off publishing "KIDOWED" for years, because I just didn’t know how it would be received, and basically I was scared that it would end up being a terrible decision, but it wasn’t, and I’ll never regret it even if it never sells another copy. It has given me the amazing opportunity to reach thousands of people that might otherwise have never heard of EB, and that’s nothing to sneeze at!

We enjoy introducing the community throughout the year to various people who are in some way involved with raising awareness or focus about EB. One such person is author and mom Jessica Kenley. Jessica lost two of her children, Ethan Tyler and Kaylee Marie, to the Junctional-Herlitz subtype of Epidermolysis Bullosa during their infancy. She later gave birth to her third child, Lilliana Marie, in 2009, who has since shown no symptoms of EB.

In 2012, Jessica decided to share her own EB story by writing and publishing her book, KIDOWED.

Her account of her family’s experience with EB has been described as honest, stark and brave. Her decision to tell the story through a “no holds barred” approach has been met with mixed feedback because of its strong language. We reached out to her recently to get her thoughts on what her process was in sharing her story, what the experience has been like interacting with other EB families and others in the community and how she has handled receiving both positive and negative feedback from others in doing so.

In this first blog of the two-part interview, Kenley discusses her motivation behind writing the book and the responses to it.

Q. EB Resource (EBR): You detail in KIDOWED what it has been like as a parent to experience the devastation of the loss of two children to EB. For those of our readers who have not yet read the book, what compelled you to write your own book and share such a deeply personal experience with others?
A. Jessica Kenley (Jessica): First and foremost, I wanted more people to know about EB, because I was shocked that so few people had ever heard of it, especially that the doctors — even the pediatric dermatologists that I took my firstborn Ethan to — had virtually no experience with it, and those who had had never treated the subtype that my children had. This is a disease that has devastating effects, a high mortality rate, and is just plain horrible to be afflicted with, yet I had never heard of it, and the vast majority of the people that I talked to hadn’t either. It was shocking.

Secondly, I knew that there had to be more people out there like me — and by that I mean people who had gone through a terrible experience and were unable to find the good in it for a very long time, if at all. I was extremely angry for many years, and still am sometimes, about losing my first two children in such a horrific way while I stood by seemingly helpless. I was not and am not religious, and was not comforted at all by the fact that people told me that they were “in a better place” or “went to be with God.” All I knew was that they were supposed to be here on Earth with me, and it just seemed incredibly unfair, especially with the odds that I faced of even having a child with EB, that they first of all had the only known universally fatal subtype, and secondly that they both were afflicted with it with only a 25% chance of the genes matching up that way with each pregnancy. I was just furious, and I knew I couldn’t be the only one, considering that anger is one of the documented phases of grief.

Q. EBR: When you told others of your plan to write the book, what kind of response did you get about doing it?
A. Jessica: Overall, I got a fairly good response. People in my family and friend circle thought that it was a decent idea, although I did have several people proofread it, and one woman who is a therapist said that she couldn’t put it down, but at the same time wanted to “throw it across the room.” I knew that the book would not be easy emotionally for anyone to read, but I still felt like it was very important to publish it. A couple people recommended that instead of publishing it, I should instead start a blog to see how that went, but that didn’t make a lot of sense to me, because I figured I could reach more people if I published it, and I also didn’t have an endless amount of things to write about after Kaylee died. I didn’t want the book to be mostly about me, I wanted it to be mostly about the children and my experience as a single mother to two children with EB, and not so much about my experience after they died, because I am certainly not an expert on grief or on how to successfully continue with one’s life after their children have died.

Q. EBR: When you set out to write the book, what objectives did you have in mind to get the story out there?
A. Jessica: To be quite honest, my only objective was to try to spread awareness about Epidermolysis Bullosa, and since I am not a medical professional, I couldn’t really write a whole lot about the science or medicine behind it and expect people to take me seriously, so I wrote about what I knew, which was my feelings and experiences I had while I was the mother of two children with EB. The book is simply my journal, and I started writing it as if I was talking to another person just because I was trying to justify the crazy thoughts in my head to myself at the time, if that makes any sense. I didn’t know when I started writing it if I ever would actually publish it, but I knew that if I did, it would have to be very honest, because I think that is the best way to reach out to and relate to other people.

Q. EBR: You are quite candid in the book both with your honesty about the conversations and interactions you describe but also in the language that you use throughout the book. Why was that important to you in the telling of the story?
A. Jessica: It was important to me to convey what I was actually feeling and thinking at the time, even if it was irrationally angry or sort of emotionally over the top, and even if that meant using a lot of curse words, italics/bold/capital letters. I think it’s difficult to express very strong emotions and tone in the written word sometimes, and I wanted people who were reading it to feel like this is how it would sound if it were actually coming out of my mouth if we were having a conversation. I didn’t have a good “sounding board” at the time I was writing the book, someone I could say anything to, so I guess that what I wrote is how I would have been speaking if I were talking to my best friend. I also didn’t want the book to be “sugar-coated” at all or censored in any way, because I felt at the time like this would have made it less “real” and people wouldn’t relate to it as well. The bottom line is that most people have had thoughts like the ones I wrote down, even if they don’t say them out loud, and I didn’t feel like a diary should necessarily be required to be polite.

Q. EBR: So what kind of feedback have you received from others so far?
A. Jessica: The majority of the feedback about the book has been good, especially from people who went through a similar experience as me. I do, however have a few 1-star and 2-star reviews on Amazon.com, and several people in my family and in my town who were portrayed in an unflattering light were extremely angry with me. Some felt as if their privacy had been violated, and others were embarrassed about what I wrote about them. The people who don’t know me and criticized the book did so by attacking my personal life choices, such as choosing to have two more children after my son died.  I have apologized extensively to the people who were hurt unintentionally and contacted me about it, both publicly and personally. My intentions for this book were certainly not to sling mud, but to convey my feelings about a horrible situation that I was trying to survive with admittedly poor coping skills.  The rest of the people who contacted me and had positive feedback have been many, and I try to focus on that.

Because of the book, I am friends with an entire new group of people related to the EB community that I would have never known had I not published it, and for that I am extremely grateful.  Many people have moved me to tears with their heartfelt messages about how they could identify with my feelings of anger and helplessness, and several have told me that they were so glad that I published it because they felt like they were the only ones who felt similar to the way I did after a loved one died, as if they were abnormal and it was socially unacceptable for them to express those feelings, because the push in our culture really is for people to “move on” and “find the good” in the situation.

Q. EBR: In the book, you talk about the expectations people had of you as a parent and the various ways they responded to you and your children. What can you share with our readers about that aspect of dealing with EB, something that really stood out from your experience that perhaps parents of newly diagnosed children could learn from?
A. Jessica: It was very apparent with me right from the get-go that I would have to develop a pretty thick skin about my decisions involving Ethan and Kaylee’s care, especially some of the controversial medical decisions I was making, such as narcotics at an early age and resisting invasive treatments like g-tubes, dilations and even blood transfusions. So few people have ever heard of EB, including the medical community, that parents of newborns with EB are required to become instant experts in order to care for their children well. My advice for parents of newly diagnosed children would be this: TRUST YOURSELF! No matter what everyone else’s advice is, and whether it is asked for or not, you will receive a whole lot of it, but you will know what works and what doesn’t for your child, and what is best for them.

Also, I definitely did not do this, but I would recommend coming up with some “canned responses” to the very insensitive and offensive questions that people ask, because it certainly didn’t do me any good to be nasty about it. These would include things like when people say, “Oh, so he/she just has sensitive skin, right?” and then launch into a story about how they had to buy special laundry detergent or something for their child in an effort to relate to your situation. I found that if I tried to explain the mechanics of the disease to these sorts of people, their eyes would glaze over and they wouldn’t pay attention anyway. It just seemed that they wanted to talk about themselves, which irritated me to no end. The bottom line is that there are only so many people that are willing to be educated, and the rest are really not worth your anger or frustration, because it certainly won’t do you or your child any good.

Next week, we will feature part two of the Q&A with Jessica when she talks further about other outcomes from writing the book and needs within in the EB community.